Raras
Buscar doenças, sintomas, genes...
Hipotensão intracraniana espontânea
ORPHA:443180CID-10 · G96.0CID-11 · 8D61.0DOENÇA RARA

A doença de Moyamoya (DMM) é uma doença cerebrovascular crónica e oclusiva caracterizada por estenose progressiva na porção terminal da artéria carótida interna e por uma rede vascular anormal na base do cérebro. Embora a sua etiologia permaneça desconhecida, estudos genéticos recentes identificaram o RNF213 na região 17q25-ter como um importante gene de susceptibilidade da DMM nas populações da Ásia Oriental. Possivelmente devido a diferenças genéticas, a DMM é relativamente comum em pessoas que vivem em países da Ásia Oriental, como a Coreia e o Japão, em comparação com as do Hemisfério Ocidental. A prevalência da DMM parece ser ligeiramente inferior entre os chineses, em comparação com os coreanos ou japoneses.

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Introdução

O que você precisa saber de cara

📋

Dor de cabeça postural intensa, piora em pé e melhora ao deitar. Pode haver náuseas, vômitos e zumbido. Causada por perda de líquido cefalorraquidiano sem trauma aparente.

Pesquisas ativas
5 ensaios
10 total registrados no ClinicalTrials.gov
Publicações científicas
1.561 artigos
Último publicado: 2026 Apr 13

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
5.0
Worldwide
Início
Adolescent
+ adult, childhood, elderly
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: G96.0
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.561PubMed
Últimos 10 anos200publicações
Pico2025138 papers
Linha do tempo
2026Hoje · 2026🧪 2014Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico8
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 8 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Hipotensão intracraniana espontânea

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Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

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Ensaios clínicos abertos e novidades científicas recentes

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Outros ensaios clínicos

10 ensaios clínicos encontrados, 5 ativos.

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Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
970 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 970

#1

Technique of performing an epidural blood patch in spontaneous intracranial hypotension due to cerebrospinal fluid leakage from the nerve root sheath: Case series.

Clinical neurology and neurosurgery2026 Mar 18

Spontaneous intracranial hypotension (SIH) is frequently caused by cerebrospinal fluid leak throughout dural tear in spinal nerve sheath which effects in disruption of intracranial homeostasis. We present a method of sealing that defect with epidural blood patch which is administrated via intervertebral foramen using computer tomography (CT) guidance to maximize the procedure accuracy. The method involves sealing CSF fistulae in spinal nerve sheaths using route via intervertebral foramen under constant CT guidance with patient's own venous blood. Technique has been used in the group of 9 patients. Clinical symptoms for spontaneous intracranial hypotension ended up in all patients. Also, follow-up MRI scans performed at 1 months after procedure showed a normalization of radiological image. Our study shows safe and effective way of sealing dural defects in spinal nerve sheath which was the cause of SIH.

#2

Pituitary height and clivus-mesencephalic angle in combination with Bern Score in the diagnosis of spontaneous intracranial hypotension.

Brain research bulletin2026 Feb 26

There is a lack of reliable spontaneous intracranial hypotension (SIH) diagnosing tools. The commonly used Bern Score, which integrates six established imaging signs, has proven reliable in discriminating spinal cerebrospinal fluid (CSF) leaks. However, this score does not include several commonly observed imaging features, and new signs have been reported recently. This study aimed to identify additional reliable imaging signs in order to develop a new scoring system for the diagnosis of SIH. In this case-control study, brain MR images from consecutive SIH patients and control participants at our institution were retrospectively and independently reviewed by 3 blinded readers. 3 qualitative and 9 quantitative imaging signs were evaluated. Firth's regression was performed and a diagnostic scoring system composed of reliable imaging signs was developed. The performance of this new scoring system was validated in an external retrospective patient cohort and compared with that of the Bern Score. Our derivation cohort comprised 20 SIH patients and 20 age- and gender- matched control participants, while the validation cohort included 55 SIH patients and 55 controls. We identified pituitary height (ICC=0.873) and the clivus-mesencephalic angle (ICC=0.897) as two additional reliable and practicable imaging features. All six imaging signs in the original Bern Score showed good inter-observer and inter-study agreement (except for prepontine cistern size, which was smaller in the present study). We then developed a new scoring system, the PS-Bern score (PS denoting pituitary and skull base). It incorporates pituitary height (2 points), clivus-mesencephalic angle (1 point) and the full Bern Score (prepontine cistern size [≤4 mm]). In the validation cohort, the PS-Bern score outperformed the Bern score. For ruling in SIH (cutoff ≥5 vs Bern ≥4), the PS-Bern score had a sensitivity of 89.1% and specificity of 98.2%, compared to 85.5% and 94.6% for the Bern score. For ruling out SIH (cutoff ≤3 vs Bern ≤2), its sensitivity and specificity were 89.1% and 92.7%, respectively, superior to the Bern score's values of 65.5% and 90.9%. Pituitary height and clivus-mesencephalic angle are useful imaging biomarkers and the PS-Bern score including these two signs may aid in the timely and accurate diagnosis of SIH. The small sample size requires that the results be validated in larger cohorts.

#3

Return to work - estimated socioeconomic impact of spontaneous intracranial hypotension and effects of neurosurgical treatment.

Frontiers in neurology2026

Spontaneous intracranial hypotension (SIH) due to a spinal cerebrospinal fluid (CSF) leak is a debilitating but curable condition, often affecting individuals of working age, making a considerably socioeconomic impact likely. This monocentric retrospective study in Germany analyzed work capacity in patients ≤65 years before and after surgical closure of a spinal CSF leak between April 2018 and September 2024. The economic burden was evaluated via direct hospital costs and indirect costs from productivity losses. Two hundred and ten patients (median age 45.5 years, 62% female), all physically capable of working, were included. After symptom onset, 96% could not perform their professional work as before: 61% were completely unable, 18% reduced working time, and 17% adapted conditions. Three months postoperatively, 55% had completely returned to work, 19% were working part-time. At the last follow-up these numbers further improved to 65 and 17%, respectively, only 9% were still unable to work, 9% had retired. A shorter symptom duration was significantly associated with complete return to work. Median direct costs per patient for diagnosis and treatment was €11,407, indirect costs for 160 days (the median symptom duration before surgery) averaged €21,169. Extrapolated to the incidence rate, the additional annual economic burden in Germany is estimated at €85.75 million for 160 sick days, largely from preventable productivity losses. SIH significantly impairs working ability. Early treatment can restore work capacity and substantially reduces preventable productivity losses, strongly advocating timely intervention not only from a medical but also from an economic perspective.

#4

Spontaneous Intracranial Hypotension in the Postpartum Period: A Case Report.

Neurology India2026 Jan 01

Spontaneous intracranial hypotension (SIH) is characterized by an orthostatic variation in headache precipitation.The typical history connotes headaches that are triggered by an upright posture and partially or completely relieved in the supine decubitus. In most patients, the site of cerebrospinal fluid (CSF) leak is at the cervical or thoracic spinal level. The imaging modalities needed to establish the diagnosis of SIH include computed tomography (CT) and magnetic resonance imaging (MRI) of the brain, CT, and MRI myelography, and radionuclide cisternography. Treatment usually consists of conservative measures of prolonged bedrest in the supine decubitus over a few weeks. However, patients unresponsive to such measures are treated by an autologous epidural blood patch (EBP) administration at the site of CSF leak. We describe an unusual patient who developed SIH due to vigorous bearing down efforts during childbirth resulting in dural tear at cervical spine level and who was treated with a minimally invasive percutaneous image guided epidural blood patch using freshly drawn autologous blood with a very rewarding outcome.

#5

Symptom Relief and Quality of Life in Patients Managed for Spontaneous Intracranial Hypotension.

Neurology India2026 Jan 01

Spontaneous intracranial hypotension (SIH) results from cerebrospinal fluid loss, mostly due to a spinal dural tear. Patients with SIH are treated with an autologous epidural blood patch (EBP) if conservative treatment fails. There is a paucity of studies from India regarding the symptom relief and long-term outcomes of patients with SIH. In this study, we present our institutional experience regarding the clinical-epidemiological profile, imaging findings, and long-term outcomes of patients after treatment of SIH who have presented to our hospital since 2012. We retrospectively examined the medical records of all patients with a diagnosis of SIH from 2012 to date. We collected data regarding the clinical presentation, imaging findings, and treatment received. Subsequently, we performed telephonic follow-ups to enquire about their headache severity at 3 months and 6 months (using a numeric rating scale) after treatment, current quality of life by using the EuroQOL-5D questionnaire, and sleep quality. Since 2012, 37 SIH patients have been treated at our institution. The predominant symptom was headache (97%), and 86% had postural headache. Pachymeningeal enhancement, engorged venous sinuses, subdural collection, and spinal longitudinal epidural collection were the most common findings on magnetic resonance imaging (MRI), with a median Bern score of 9. Twenty-five (68%) patients received EBP, most commonly at the C6-C7 level, mostly with fluoroscopic assistance. About 71% of patients achieved complete symptom relief. Post-EBP MRI (n = 12) showed partial resolution of SIH features. On telephonic follow-up, the median headache score at 3 and 6 months was 3. The median QOL scores were 1 for mobility, self-care, usual activity, pain, and anxiety/depression, implying no problems in these dimensions. The median sleep score was 100, indicating excellent sleep. Patients have good symptom relief following treatment of SIH, leading to a good quality of life, even years after the initial presentation.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC1.106 artigos no totalmostrando 196

2026

Intracranial hypotension secondary to a spontaneous cervical nerve root leak repaired using a chimeric rotational paraspinal flap: a multidisciplinary surgical approach. Illustrative case.

Journal of neurosurgery. Case lessons
2026

Resolution of spontaneous intracranial hypotension due to a spinal CSF-venous fistula after a dynamic myelogram: illustrative case.

Journal of neurosurgery. Case lessons
2026

Technique of performing an epidural blood patch in spontaneous intracranial hypotension due to cerebrospinal fluid leakage from the nerve root sheath: Case series.

Clinical neurology and neurosurgery
2026

Prone Digital Subtraction Myelography versus Dynamic CT Myelography for Detecting Definite Type 1 CSF Leak in Spontaneous Intracranial Hypotension.

AJNR. American journal of neuroradiology
2026

Temporal and regional trends in the diagnosis and treatment of spontaneous intracranial hypotension: a systematic review.

The journal of headache and pain
2026

Sacral Dural Tears in Spontaneous Intracranial Hypotension: Imaging Phenotype and Treatment Outcomes in a Multicenter Cohort.

AJNR. American journal of neuroradiology
2026

Audiovestibular manifestations of intracranial hypotension: a descriptive clinical series.

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
2026

Symptomatic Spontaneous Intracranial Hypotension Caused by a Cerebrospinal Fluid Leak at the C1-C2 Level of the Spine Sealed With an Epidural Blood Patch-Case Series and Review of Literature.

Pain practice : the official journal of World Institute of Pain
2026

Art of imaging: brain sagging in spinal cerebrospinal fluid leak.

Radiology advances
2026

Low interstitial fluid in patients with spontaneous intracranial hypotension.

Fluids and barriers of the CNS
2026

Management of spontaneous intracranial hypotension during second trimester of pregnancy and neuraxial labour analgesia for operative vaginal delivery: a case report.

International journal of obstetric anesthesia
2026

Occurrence of CSF-Venous Fistulas by Spinal Level and Laterality: A Systematic Review and Meta-Analysis.

AJNR. American journal of neuroradiology
2026

Pituitary height and clivus-mesencephalic angle in combination with Bern Score in the diagnosis of spontaneous intracranial hypotension.

Brain research bulletin
2026

Prediction for occurrence of subdural hematoma following spontaneous intracranial hypotension: a large cohort of 575 adults in a single center from 2023 to 2024.

The journal of headache and pain
2026

Spinal CSF Volumetry in Patients with Spontaneous Intracranial Hypotension and Spinal CSF Leaks.

AJNR. American journal of neuroradiology
2026

Non-targeted thoracic epidural blood patch for spontaneous intracranial hypotension complicated by bilateral subdural haemorrhages in a patient with end-stage renal failure: case based review.

Irish journal of medical science
2026

Does change of spinal diverticular size predict the site of de novo recurrent CSF-venous fistulas in patients with spontaneous intracranial hypotension?

AJNR. American journal of neuroradiology
2026

Ultrasound measurement of optic nerve sheath diameter pre and post lumbar puncture for prediction of postdural puncture headache.

Scientific reports
2026

CT-guided cyanoacrylate injection for spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension: outcomes from an expanded cohort.

Journal of neuroradiology = Journal de neuroradiologie
2026

Commentary: Minimally Invasive Transdural Closure of a Ventral Spinal Cerebrospinal Fluid Leak in Spontaneous Intracranial Hypotension: 2-Dimensional Operative Video.

Operative neurosurgery (Hagerstown, Md.)
2026

Resolution of pachymeningeal enhancement is a radiographic marker for effective treatment of spontaneous intracranial hypotension: patient series.

Journal of neurosurgery. Case lessons
2026

Return to work - estimated socioeconomic impact of spontaneous intracranial hypotension and effects of neurosurgical treatment.

Frontiers in neurology
2026

Imaging of CSF-Venous Fistulas at the High and Low Ends of the Spine: Techniques and Case Examples.

AJNR. American journal of neuroradiology
2026

Chiari Decompression in Patients with Spontaneous Intracranial Hypotension: Presenting Symptoms and Treatment Response.

AJNR. American journal of neuroradiology
2026

Safety and Efficacy of Intracystic Fibrin Glue Injection for CSF-Venous Fistulas.

AJNR. American journal of neuroradiology
2026

The impact of human brain geometry on the transport of an intrathecal tracer.

Fluids and barriers of the CNS
2026

Spontaneous Intracranial Hypotension in the Postpartum Period: A Case Report.

Neurology India
2026

Symptom Relief and Quality of Life in Patients Managed for Spontaneous Intracranial Hypotension.

Neurology India
2026

A Nomogram to Predict of Epidural Blood Patch Treatment Failure in Patients With Spontaneous Intracranial Hypotension and Subdural Hematoma.

Pain physician
2026

The SIH Impact Inventory: A Pilot Study of a Novel Instrument Assessing Quality of Life in Spontaneous Intracranial Hypotension.

Neurology. Clinical practice
2026

Epidural Onyx Treatment for Spontaneous Intracranial Hypotension Caused by a Ventral Dural Tear.

AJNR. American journal of neuroradiology
2026

Reversible amnestic syndrome due to spontaneous intracranial hypotension: A case of bi-hippocampal herniation.

Revue neurologique
2026

Central Venous Sinus Thrombosis with Spontaneous Intracranial Hypotension: A Case Report.

Acta neurologica Taiwanica
2026

Endovascular treatment of a sacral cerebrospinal fluid-venous Fistula using transvenous embolization: A case report.

Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences
2025

Continuous epidural saline infusion for spontaneous intracranial hypotension with cortical vein thrombosis: illustrative case.

Journal of neurosurgery. Case lessons
2026

Obstructive hydrocephalus secondary to brainstem sagging in recurrent cerebrospinal fluid-venous fistulas: a diagnostic and therapeutic challenge. Illustrative case.

Journal of neurosurgery. Case lessons
2026

The Importance of Small Lateral Dural CSF Collections in Spontaneous Intracranial Hypotension: A Radiologic-Anatomic Study.

AJNR. American journal of neuroradiology
2026

Detection of Cerebrospinal Fluid-venous Fistulas Using Digital Subtraction Myelography in Refractory Spontaneous Intracranial Hypotension: A Single-center Study in an Asian Population.

Neurologia medico-chirurgica
2026

CSF-venous fistulas Reconsidered: Pressure paradox and the Volume-Elastance Relationship.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2025

Intracranial hypotension headache complicated by retroclival subdural hematoma: clinical insights and literature review.

Frontiers in neuroscience
2026

Superior vena cava obstruction causing diffuse pachymeningeal enhancement mimicking spontaneous intracranial hypotension.

Journal of neurology
2025

Parasagittal subdural space: a novel quantitative marker of spontaneous intracranial hypotension syndrome-induced chronic subdural hematoma.

BMC medical imaging
2025

Progressive Bilateral Sensorineural Hearing Loss and Ataxia Caused by Superficial Siderosis Secondary to Chronic Spontaneous Intracranial Hypotension.

Cureus
2025

[Otologic symptoms and audiological characteristics in patients with spontaneous intracranial hypotension].

Zhonghua yi xue za zhi
2025

Idiopathic CSF dynamic disturbances causing headache: a narrative review.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2025

No increased risk of spinal cerebrospinal fluid leak after spinal manipulative therapy: A retrospective cohort study.

PM &amp; R : the journal of injury, function, and rehabilitation
2025

Successful streamlined photon-counting CT myelography protocol for detection of CSF-venous fistulas in busy practice settings.

AJNR. American journal of neuroradiology
2025

Occult spinal CSF leak: to patch or not to patch-a case-based update.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Spontaneous Intracranial Hypotension: A Commonly Missed Cause of Secondary Headache.

Cureus
2025

Spontaneous Intracranial Hypotension Presenting as Cryptogenic Coma: A Case Report and Literature Review Emphasizing the Role of Advanced Myelography.

Cureus
2025

The Association of Spinal Meningeal Diverticula Number and Size with the Detection of CSF-Venous Fistulas: Evidence from a Contemporary Myelography Cohort.

AJNR. American journal of neuroradiology
2025

Direct Surgical Repair of Spontaneous Spinal CSF Leak: A Retrospective Canadian Cohort and Systematic Literature Review.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2025

An Unusual Case of Spontaneous Intracranial Hypotension Presenting as Meningoencephalitis.

European journal of case reports in internal medicine
2025

Neuroimaging with photon-counting computed tomography: A review of clinical applications.

World journal of radiology
2025

A Case of Burr Hole Drainage and Middle Meningeal Artery Embolization for Chronic Subdural Hematoma Associated with Spontaneous Intracranial Hypotension.

Asian journal of neurosurgery
2025

Simultaneous Burr Hole Drainage and Epidural Blood Patch with Real-Time ICP Monitoring for Subdural Hematoma Associated with Spontaneous Intracranial Hypotension: Case Report and Technical Note.

Asian journal of neurosurgery
2025

Associations of Refractory Spontaneous Intracranial Hypotension and Chronic Subdural Hematoma with Factor XIII Deficiency: A Case Report.

Asian journal of neurosurgery
2025

A dural displacement sign in patients with type 1 cerebrospinal fluid leakage.

Neuroradiology
2025

Rates of CT myelography epidural contrast extravasation at puncture site by spinal needle type.

Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences
2025

Spontaneous Intracranial Hypotension in Pregnancy with Aggravated Comorbidity: A Case Report and Review of Diagnostic and Management Challenges.

Reports (MDPI)
2025

Duropathies: A Narrative Overview of a Neglected Concept-Part One: Anatomical, Embryological, and Pathophysiological Elements.

NeuroSci
2025

Consensus Guidelines on Diagnostic Brain and Spine Imaging of Spontaneous Intracranial Hypotension.

AJNR. American journal of neuroradiology
2025

The Role of Trendelenburg Positioning for the Acute Symptomatic Management of Spontaneous Intracranial Hypotension.

The Neurohospitalist
2025

Beyond the Headaches: Severe Dysphagia As the Dominant Symptom of Spontaneous Intracranial Hypotension.

Cureus
2025

Spontaneous Intracranial Hypotension: A Rare Etiology of Subarachnoid Hemorrhage.

Cureus
2025

Diagnostic analysis of a new sign (superior sagittal sinus distention) on spontaneous intracranial hypotension MR imaging: a retrospective study.

BMC medical imaging
2025

Through Both Lenses: A Dual Perspective on Illness.

Journal of patient experience
2025

Coma from severe spontaneous intracranial hypotension due to a CSF-venous fistula.

Practical neurology
2025

Incidence and Predictors of Rebound Intracranial Hypertension After Transvenous Embolization of Cerebrospinal Fluid-Venous Fistula.

AJNR. American journal of neuroradiology
2025

Comparison of Suprasellar Cistern Height Measurements Between Coronal and Sagittal Planes.

World neurosurgery
2025

Headaches due to Low and High Intracranial Pressure.

Physical medicine and rehabilitation clinics of North America
2025

Neuroimaging and Diagnostic Workup for Headache.

Physical medicine and rehabilitation clinics of North America
2025

Endovascular Embolization Techniques for Cerebrospinal Fluid-Venous Fistula in the Treatment of Spontaneous Intracranial Hypotension.

Neurosurgery clinics of North America
2025

Percutaneous Treatment and Post-treatment Management of CSF Leaks and CSF-Venous Fistulas in Spontaneous Intracranial Hypotension.

Neurosurgery clinics of North America
2025

Spinal Cerebrospinal Fluid Leak Localization with Digital Subtraction Myelography: Tips, Tricks, and Pitfalls.

Neurosurgery clinics of North America
2025

Spinal Cerebrospinal Fluid Leak Localization with Dynamic Computed Tomography Myelography: Tips, Tricks, and Pitfalls.

Neurosurgery clinics of North America
2026

Recommendations for the diagnosis and treatment of spontaneous intracranial hypotension.

La Radiologia medica
2025

Atypical Imaging Manifestations of Lateral Dural Tears with Arachnoid Herniations: A Multi-Institutional Study.

AJNR. American journal of neuroradiology
2025

Critical Reappraisal and Validation of the Bern Score System for Diagnosing Spontaneous Intracranial Hypotension.

Journal of clinical neurology (Seoul, Korea)
2025

Unraveling the cause of microspurs in spontaneous intracranial hypotension type 1: discogenic origin or calcified Hofmann's ligament?

Journal of neurosurgery. Spine
2026

Lateral ventricular choroid plexus enlargement in patients with spontaneous intracranial hypotension.

Headache
2025

[Myelography-still up to date?].

Radiologie (Heidelberg, Germany)
2025

Navigating the epidural plexus: a venographic classification system to predict technical feasibility in cerebrospinal fluid-venous fistula embolization.

Journal of neurointerventional surgery
2026

The contribution of lumbar puncture opening pressure in the diagnosis of spontaneous intracranial hypotension: A systematic literature review and meta-analysis.

Headache
2026

Spine MRI Diverticular Patterns Predict CSF-Venous Fistula Location: A 100-Patient Study.

AJNR. American journal of neuroradiology
2025

Microcatheter Intrathecal Selective Myelography for Intraprocedural CSF-Venous Fistula Evaluation Pre-and Post-Embolization.

AJNR. American journal of neuroradiology
2025

CSF-venous Fistulas Occurring in First Degree Relatives: A Multi-Center Case Series.

AJNR. American journal of neuroradiology
2026

Temporal Characteristics of Type 2 Lateral Spinal CSF Leaks on Digital Subtraction Myelography: Fast, Medium, or Slow Leaks?

AJNR. American journal of neuroradiology
2024

GRADUAL SPONTANEOUS REGRESSION OF A LARGE PITUITARY MASS - SHOULD WE TRUST THE TIME TO HEAL SOME WOUNDS?

Acta endocrinologica (Bucharest, Romania : 2005)
2026

Locating Spinal Leaks in Spontaneous Intracranial Hypotension: How Many Dynamic Myelographies Does It Take?

AJNR. American journal of neuroradiology
2025

Disparities in Access to Specialized Care for Spontaneous Intracranial Hypotension.

AJNR. American journal of neuroradiology
2026

Spontaneous Intracranial Hypotension and CSF-Venous Fistulae: from Diagnostic Imaging to Interventional Management.

Clinical neuroradiology
2026

Atypical presentation of a severe form of spontaneous intracranial hypotension due to multiple CSF leaks in the brain and spinal cord: therapeutic challenge.

Acta neurologica Belgica
2025

AI-Augmented Quantitative MRI Predicts Spontaneous Intracranial Hypotension.

Diagnostics (Basel, Switzerland)
2025

Middle meningeal artery embolization as an alternative strategy for symptomatic CSF hypovolemia-related chronic subdural hematoma: A case series.

Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences
2025

CSF pressures in spontaneous intracranial hypotension due to CSF-venous fistula: A retrospective analysis.

Clinical neurology and neurosurgery
2026

Patients with chronic post-dural puncture headache do not have typical imaging features of intracranial hypotension: An MRI study using the Bern score.

Headache
2025

Spontaneous Intracranial Hypotension Complicated by Bilateral Cerebral Venous Thrombosis in an Older Adult: A Case Report.

Cureus
2026

The Diagnostic Burden of Spontaneous Intracranial Hypotension: Imaging Volume and Specialists' Involvement prior to Diagnosis.

AJNR. American journal of neuroradiology
2025

Chronic Subdural Hematoma Masquerading as Intracranial Hypotension.

Cureus
2025

Syrinx in spontaneous intracranial hypotension treated by surgical disconnection of CSF venous fistula.

Practical neurology
2025

Percutaneous CT-guided Onyx embolization of CSF-venous fistula: illustrative case.

Journal of neurosurgery. Case lessons
2026

Improved Conspicuity of CSF-Venous Fistulas with Saline Pressure Augmentation: A Multi-Institutional Case Series.

AJNR. American journal of neuroradiology
2026

Maximizing the Conspicuity of CSF-Venous Fistulas on CT Myelography: Assessment of Contrast Density and Timing Effects.

AJNR. American journal of neuroradiology
2025

Headache due to Spontaneous Intracranial Hypotension in a Patient with Vertebral Bone Metastasis.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2025

Quality of life in patients with spontaneous intracranial hypotension: A systematic review and meta-analysis.

Revue neurologique
2025

Headache After Sealing of Cerebrospinal Fluid Leaks in Patients With Spontaneous Intracranial Hypotension.

European journal of neurology
2025

Posterior fossa subdural hematoma in spontaneous intracranial hypotension.

The American journal of emergency medicine
2025

Prioritizing clinical indicators over radiological findings in the management of chronic subdural hematoma associated with spontaneous intracranial hypotension.

Neuro-Chirurgie
2024

Difficult Diagnosis of Spontaneous Intracranial Hypotension with Nausea and Lower Abdominal Pain as Main Complaints: A Case Report.

Reports (MDPI)
2025

Catheterization techniques and outcomes of transvenous embolization of cerebrospinal fluid-venous fistulas in the management of spontaneous intracranial hypotension: case series.

Journal of neurointerventional surgery
2025

Spontaneous Intracranial Hypotension.

The New England journal of medicine
2025

The utility of preoperative computed tomography-guided screw marking in thoracic spine surgery.

Brain &amp; spine
2025

Quantitative analysis of thecal sac volume and morphology as a diagnostic tool in intracranial hypotension.

Headache
2025

Management of chronic subdural hematoma in spontaneous intracranial hypotension.

Brain &amp; spine
2025

Recurrent Subdural Hematoma: A Case Report of Diagnostic Pitfall of Spontaneous Intracranial Hypotension and Successful Management With Targeted Epidural Blood Patch.

Clinical case reports
2025

Cerebrospinal fluid-venous fistula: local experience with an increasingly common entity.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2025

Minimally Invasive, Tubular Approach for Ligation of Spinal Cerebrospinal Fluid-Venous Fistulas: A Single Surgeon, Consecutive Case Series.

Operative neurosurgery (Hagerstown, Md.)
2025

Altered brain fluid dynamics in spontaneous intracranial hypotension.

Fluids and barriers of the CNS
2026

Multiple Synchronous CSF-Venous Fistulas in Spontaneous Intracranial Hypotension: A Multi-Institutional Case Series.

AJNR. American journal of neuroradiology
2025

Irregular Cervicothoracic Nerve Sheaths on Digital Subtraction Myelography: A CSF-Venous Fistula Mimic.

AJNR. American journal of neuroradiology
2025

Targeted Versus NonTargeted Epidural Blood Patch for Spontaneous Intracranial Hypotension: A Systematic Review and Meta-Analysis.

European journal of neurology
2025

Outcomes of CT-Guided Targeted Epidural Patching for Lateral Dural Tears in Spontaneous Intracranial Hypotension: A Multicenter Retrospective Cohort Study.

AJNR. American journal of neuroradiology
2025

Spontaneous Intracranial Hypotension and Dural Tear in a Pediatric Patient After a Minor Fall: A Case Report.

The Journal of emergency medicine
2025

Systematic Review of the Predictive Value of Negative Brain or Low Probability Brain MRIs in Patients with CSF-Venous Fistulas.

AJNR. American journal of neuroradiology
2025

Simulation of cerebrospinal fluid (CSF)-venous fistula embolization in a swine model: A technical video.

Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences
2026

Transdural Approach for Repair of a Spontaneous Ventral Thoracic Cerebrospinal Fluid Leak Using Muscle Plug: 2-Dimensional Operative Video.

Operative neurosurgery (Hagerstown, Md.)
2026

Intradural venous engorgement of CSF-venous fistula mimics spinal dural arteriovenous fistula on MRI: A novel case report and review of literature.

The neuroradiology journal
2026

Minimally Invasive Transdural Closure of a Ventral Spinal Cerebrospinal Fluid Leak in Spontaneous Intracranial Hypotension: 2-Dimensional Operative Video.

Operative neurosurgery (Hagerstown, Md.)
2025

Twist-drill craniostomy with pressure-controlled fibrinolytic irrigation therapy reduces recurrence of chronic subdural hematomas: Could spontaneous intracranial hypotension be more common during chronic subdural hematomas than we diagnose?

Clinical neurology and neurosurgery
2025

Surgical Treatment of Spontaneous Intracranial Hypotension From Spinal Cerebrospinal Fluid Leak: Single Institution Case Series.

Neurosurgery
2025

Commentary: Surgical Treatment of Spontaneous Intracranial Hypotension From Spinal Cerebrospinal Fluid Leak: Single Institution Case Series.

Neurosurgery
2025

SPECT/CT as Gatekeeper for Extrathecal Administration on 99mTc-DTPA Cisternography.

Clinical nuclear medicine
2025

Treatment of Persistent Headache After Normalization of CSF Pressure.

Continuum (Minneapolis, Minn.)
2025

Treatment of Spinal CSF Leaks and Fistulas.

Continuum (Minneapolis, Minn.)
2025

Radiographic Evaluation of Spontaneous Intracranial Hypotension.

Continuum (Minneapolis, Minn.)
2025

Clinical Features and Diagnosis of Spontaneous Intracranial Hypotension.

Continuum (Minneapolis, Minn.)
2025

Standardized spinal MRI protocol: diagnosing spontaneous intracranial hypotension and correlating with brain MRI.

European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
2025

Decubitus Myelography for Spinal Extradural Arachnoid Cyst: Better Classified as a Lateral Dural CSF Leak?

AJNR. American journal of neuroradiology
2025

Spontaneous Intracranial Hypotension Due to Cerebrospinal Fluid Rhinorrhea.

JAMA neurology
2025

Spinal CSF leaks in spontaneous intracranial hypotension: A single-institution analysis of incidence, typology and treatment outcomes.

Clinical neurology and neurosurgery
2025

Digital Subtraction Myelography for the Detection of Type 1 Spinal CSF Leaks: Evaluation of Temporal Characteristics and Diagnostic Value.

AJNR. American journal of neuroradiology
2025

Plain Language Summary Publication: Mild cognitive impairment in spontaneous intracranial hypotension and its rapid reversal by repair of a spinal cerebrospinal fluid leak.

Headache
2025

The impact of CSF venous fistula embolization on patient's quality of life, a longitudinal clinical-radiological exploration.

The journal of headache and pain
2025

Conebeam CT Myelography for the Detection of Spinal CSF Leaks.

AJNR. American journal of neuroradiology
2025

Transvenous Embolization versus Surgical Intervention for CSF Venous Fistulas: A Systematic Review and Meta-Analysis.

AJNR. American journal of neuroradiology
2025

The Role of Ferumoxytol-Enhanced MR Venography in Transvenous Embolization of CSF-Venous Fistulas.

AJNR. American journal of neuroradiology
2025

Reporting the Degree of Certainty of CSF-Venous Fistulas in Patients with Spontaneous Intracranial Hypotension: The Duke CSF-Venous Fistula Confidence Score.

AJNR. American journal of neuroradiology
2025

Transient oculomotor nerve palsy associated with spontaneous intracranial hypotension in a pediatric patient.

Proceedings (Baylor University. Medical Center)
2025

Spontaneous Intracranial Hypotension in Professional Dancers: A Case Report and Rehabilitation Strategy.

Journal of dance medicine &amp; science : official publication of the International Association for Dance Medicine &amp; Science
2025

No evidence of intracranial hypotension in persistent post-traumatic headache: A magnetic resonance imaging study.

Cephalalgia : an international journal of headache
2025

Spontaneous Intracranial Hypotension-Rare Occurrence of Simultaneous Multiple Spinal Level CSF Leaks.

Neurology India
2025

Lower spinal levels and male sex are associated with greater epidural blood patch volume in spontaneous intracranial hypotension.

The journal of headache and pain
2025

Histopathologic Analysis of 5 Patients with CSF-Venous Fistulas after Surgical Nerve Root Ligation and Resection.

AJNR. American journal of neuroradiology
2025

Reversal of Coma With Trendelenburg Position in Spontaneous Intracranial Hypotension.

Mayo Clinic proceedings
2025

Repair of a ventral cervical cerebrospinal fluid leak via a single-level anterior cervical discectomy and fusion without corpectomy: illustrative case.

Journal of neurosurgery. Case lessons
2025

A rare and unusual case of spontaneous intracranial hypotension in a woman with congenital left hemipelvic dysplasia.

Radiology case reports
2025

Dural Arteriovenous Fistulas in Spontaneous Intracranial Hypotension.

Neurology India
2025

Photon-Counting CT Myelography for the Detection of Spinal CSF Leaks.

AJNR. American journal of neuroradiology
2025

Spontaneous Intracranial Hypotension in a Patient with Systemic Lupus Erythematosus and End-Stage Renal Failure: A Case Report and a Literature Review.

Brain sciences
2025

Revisiting a Rare Anomaly Described 25 Years Ago in the AJNR: A Journey from Pediatric Hemifacial Microsomia and Middle Cranial Fossa Aplasia to CSF-Lymphatic Fistula and Spontaneous Intracranial Hypotension as an Adult.

AJNR. American journal of neuroradiology
2025

Enhancing Clarity in Dynamic Myelography Reporting: Results of a Survey of Patients and Referring Providers Evaluating a Standardized Reporting System in the Myelographic Work-Up of Patients with Suspected Spontaneous Intracranial Hypotension.

AJNR. American journal of neuroradiology
2025

Efficacy of Traditional Epidural Patching versus Patching within Spinal Longitudinal Extradural Collections for Ventral Dural Cerebrospinal Fluid Leaks.

Radiology
2025

Hypersensitivity Reactions to Fibrin Glue during Epidural Blood Patching.

AJNR. American journal of neuroradiology
2025

Successful Treatment of Spontaneous Intracranial Hypotension With Subdural Hematoma With Cervical Epidural Blood Patch: A Case Report.

Cureus
2025

Unveiling the superiority: single catheter multisite epidural blood patch for the treatment of spontaneous intracranial hypotension.

Neurosurgical review
2025

Three Fluoroscopy-Guided Epidural Blood Patches for the Management of Spontaneous Intracranial Hypotension.

Cureus
2025

B-waves in noninvasive capacitance signal correlate with B-waves in ICP.

Acta neurochirurgica
2025

[Comparison of the accuracy between magnetic resonance hydrography and CT myelography in diagnosing of spontaneous intracranial hypotension syndrome].

Zhonghua yi xue za zhi
2025

Diagnosis and Management of Spontaneous Intracranial Hypotension Due to a CSF Leak: A Case of Spontaneous Recovery.

Cureus
2024

Dorsolateral Transdural Surgical Management of Spontaneous Intracranial Hypotension From Ventral Dural Cerebrospinal Fluid Leaks: Case Series and Technical Report.

Operative neurosurgery (Hagerstown, Md.)
2025

Volumetric Changes of the Choroid Plexus before and after Spinal CSF Leak Repair.

AJNR. American journal of neuroradiology
2025

Evaluation of Spontaneous Intracranial Hypotension Probabilistic Brain MRI Scoring Systems in Normal Patients.

AJNR. American journal of neuroradiology
2025

Evaluation of white matter integrity by using diffusion tensor imaging in spontaneous intracranial hypotension.

Headache
2026

Early brain MRI changes following transvenous embolization of cerebrospinal fluid-venous fistulas in spontaneous intracranial hypotension.

Journal of neurointerventional surgery
2025

Long-Term Headache Outcome and Radiological Correlates in Patients With Intracranial Hypotension.

European journal of neurology
2025

Commentary: Dorsolateral Transdural Surgical Management of Spontaneous Intracranial Hypotension From Ventral Dural Cerebrospinal Fluid Leaks: Case Series and Technical Report.

Operative neurosurgery (Hagerstown, Md.)
2025

The Overlooked Diagnosis of Spontaneous Intracranial Hypotension: Insights into Cerebrospinal Fluid Leak Anatomy.

Radiology
2025

MRI and Surgical Findings Refine Concepts of Type 2 Cerebrospinal Fluid Leaks in Spontaneous Intracranial Hypotension.

Radiology
2025

Early use of targeted blood patch in spontaneous intracranial hypotension presenting with bilateral subdural hematomas and acute infarcts.

Surgical neurology international
2025

Spontaneous subdural haematoma secondary to undiagnosed metastatic gastric adenocarcinoma.

BMJ case reports
2025

Intracranial pressure monitoring in patients with spontaneous onset of orthostatic headache.

The journal of headache and pain
2025

CSF-Venous Fistulas Arising Intraosseously within Bone Remodeled by Meningeal Diverticula.

AJNR. American journal of neuroradiology
2025

Advanced Imaging of Type 2 Spinal CSF Leaks with Ultra-High-Resolution Conebeam CT Myelography.

AJNR. American journal of neuroradiology
2025

Volumetric response after closure of a spinal CSF leak in patients with spontaneous intracranial hypotension: a multicompartmental longitudinal study.

Journal of neurointerventional surgery
2025

Evidence for cellular and solute passage between the brain and skull bone marrow across meninges: A systematic review.

Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism
2025

Azygos Vein Stenosis in Frontotemporal Dementia Sagging Brain Syndrome.

AJNR. American journal of neuroradiology
2025

Prevalence and Characteristics of Microspurs in Patients with Spontaneous Intracranial Hypotension Compared with the General Population.

AJNR. American journal of neuroradiology
2025

Defining the typical characteristics of orthostatic headache in patients with spontaneous intracranial hypotension.

Cephalalgia : an international journal of headache
2025

Introduction to Digital Subtraction Myelography for CSF-Venous Fistula Detection.

AJNR. American journal of neuroradiology
2025

Intrathecal fluorescein guidance in the treatment of intracranial hypotension due to spinal CSF-venous fistula.

BMJ case reports
2025

Role of CT myelography in the diagnosis and management of spontaneous intracranial hypotension.

Clinical neurology and neurosurgery
2024

Persistent Post-dural Puncture Headaches One Year After Lumbar Puncture: A Case Report.

Cureus
2025

Severe intracranial hypotension secondary to cerebrospinal-venous fistula in a patient with remote history of spinal decompression and fusion.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2024

Pediatric Meningeal Diseases: What Radiologists Need to Know.

Tomography (Ann Arbor, Mich.)
2024

Incidental asymptomatic spinal cerebrospinal fluid leaks: single-center experience, and a presentation of seven cases.

Journal of neurointerventional surgery
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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Technique of performing an epidural blood patch in spontaneous intracranial hypotension due to cerebrospinal fluid leakage from the nerve root sheath: Case series.
    Clinical neurology and neurosurgery· 2026· PMID 41865612mais citado
  2. Pituitary height and clivus-mesencephalic angle in combination with Bern Score in the diagnosis of spontaneous intracranial hypotension.
    Brain research bulletin· 2026· PMID 41763586mais citado
  3. Return to work - estimated socioeconomic impact of spontaneous intracranial hypotension and effects of neurosurgical treatment.
    Frontiers in neurology· 2026· PMID 41695621mais citado
  4. Spontaneous Intracranial Hypotension in the Postpartum Period: A Case Report.
    Neurology India· 2026· PMID 41633638mais citado
  5. Symptom Relief and Quality of Life in Patients Managed for Spontaneous Intracranial Hypotension.
    Neurology India· 2026· PMID 41633630mais citado
  6. Recent Updates to Diagnosis and Treatment of Spontaneous Intracranial Hypotension.
    Korean J Radiol· 2026· PMID 41974534recente
  7. Surgical management of ventral epidural cerebrospinal fluid leak: a scoping review and illustrative case.
    Eur Spine J· 2026· PMID 41973205recente
  8. Spontaneous Intracranial Hypotension: A Tertiary Care Experience.
    Ann Indian Acad Neurol· 2026· PMID 41964146recente
  9. Familial occurrence and heritable connective tissue disorders in spontaneous intracranial hypotension.
    AJNR Am J Neuroradiol· 2026· PMID 41962957recente
  10. Sacral CSF-venous fistula diagnosed using single-day bilateral decubitus myelography: A rare case and a novel technical modification.
    Radiol Case Rep· 2026· PMID 41960134recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:443180(Orphanet)
  2. MONDO:0018624(MONDO)
  3. GARD:21854(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q9333215(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Hipotensão intracraniana espontânea
Compêndio · Raras BR

Hipotensão intracraniana espontânea

ORPHA:443180 · MONDO:0018624
Prevalência
1-9 / 100 000
Herança
Not applicable
CID-10
G96.0 · Fístula liquórica
CID-11
Ensaios
5 ativos
Início
Adolescent, Adult, Childhood, Elderly
Prevalência
5.0 (Worldwide)
MedGen
UMLS
C0751731
Repurposing
13 candidatos
ameziniumadrenergic receptor agonist
ephedrinevasoconstrictor
ephedrine-(racemic)angiotensin receptor antagonist
+10 outros
EuropePMC
Wikidata
Papers 10a
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